June 2022
Volume 63, Issue 7
Open Access
ARVO Annual Meeting Abstract  |   June 2022
Optical coherence tomography angiography in inherited retinal disease
Author Affiliations & Notes
  • Amy Lu
    Ophthalmology, Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States
  • Jeffrey Sundstrom
    Ophthalmology, Penn State Health Milton S Hershey Medical Center, Hershey, Pennsylvania, United States
  • Footnotes
    Commercial Relationships   Amy Lu None; Jeffrey Sundstrom None
  • Footnotes
    Support  None
Investigative Ophthalmology & Visual Science June 2022, Vol.63, 2915 – F0068. doi:
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    • Get Citation

      Amy Lu, Jeffrey Sundstrom; Optical coherence tomography angiography in inherited retinal disease. Invest. Ophthalmol. Vis. Sci. 2022;63(7):2915 – F0068.

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      © ARVO (1962-2015); The Authors (2016-present)

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Abstract

Purpose : Diagnosis of inherited retinal diseases (IRD) remains difficult as some diseases do not demonstrate obvious findings on clinical exam. Optical coherence tomography angiography (OCTA) is an emerging, non-invasive imaging modality that may provide new information regarding these diseases. However, studies involving OCTA and IRD generally involve small case series that focus on specific IRDs. Here, we perform a database search to examine global as well as disease-specific changes in OCTA in different IRDs.

Methods : A systematic review and meta-analysis was performed by searching Pubmed. Studies utilizing OCTA in IRD were included. Search results that did not utilize OCTA, did not include eyes diagnosed with IRD, not available in English, not primary research articles or case series, or did not involve human eyes were excluded.

Results : Database search identified 25458 records. After removal of duplicates, 6603 records were subjected to inclusion/exclusion based on manual review of titles and abstract. 153 articles were included for full-text review. The most common reasons for exclusion were no diagnosis of IRD and no use of OCTA. Specifically, 1142 (17%) of non-duplicated search results included eyes that were diagnosed with IRD, of which 153 (13%) these utilized OCTA imaging. 128 (84%) of studies that were included were published between 2018 and 2021. 22 different IRDs were represented after inclusion/exclusion. Most highly represented IRD diagnoses were retinitis pigmentosa, Best disease, Stargardt’s disease, choroideremia, adult onset foveomacular vitelliform dystrophy, and X-linked retinoschisis. Preliminary analysis suggests that multiple IRDs demonstrate patterns of enlarged superficial foveal avascular zone (FAZ) and decreased vessel density compared to healthy controls, and compared to earlier forms of the disease. Further, differences in FAZ and vessel density are more prominent to specific deep or superficial layers and capillary beds, and these differ between diseases.

Conclusions : OCTA has been increasingly utilized in characterizing different IRDs as well as different stages of IRD progression. OCTA has also been incorporated in initial reports describing novel IRD entities and novel mutations in known IRDs. OCTA adds new understanding to the pathology of IRD and should be considered in the workup and continued management of IRD patients.

This abstract was presented at the 2022 ARVO Annual Meeting, held in Denver, CO, May 1-4, 2022, and virtually.

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